discoid lupus erythematosus

Discoid Lupus Erythematosus

Definition

Discoid lupus erythematosus (DLE) is a disease in which coin-shaped (discoid) red bumps appear on the skin.

Description

The disease called discoid lupus erythematosus only affects the skin, although similar discoid skin lesions can occur in the serious disease called systemic lupus erythematosus (SLE). Only about 10% of all patients with DLE will go on to develop the multiorgan disease SLE.

The tendency to develop DLE seems to run in families. Although men or women of any age can develop DLE, it occurs in women three times more frequently than in men. The typical DLE patient is a woman in her 30s.

Causes and symptoms

The cause of DLE is unknown. It is thought that DLE (like SLE) may be an autoimmune disorder. Autoimmune disorders are those that occur when cells of the immune system are misdirected against the body. Normally, immune cells work to recognize and help destroy foreign invaders like bacteria, viruses, and fungi. In autoimmune disorders, these cells mistakenly recognize various tissues of the body as foreign invaders, and attack and destroy these tissues. In SLE, the misdirected immune cells are antibodies. In DLE, the damaging cells are believed to be a type of white blood cell called a T lymphocyte. The injury to the skin results in inflammation and the characteristic discoid lesions.

In DLE, the characteristic skin lesion is circular and raised. The reddish rash is about 5-10 mm in diameter, with the center often somewhat scaly and lighter in color than the darker outer ring. The surface of these lesions is sometimes described as "warty." There is rarely any itching or pain associated with discoid lesions. They tend to appear on the face, ears, neck, scalp, chest, back, and arms. As DLE lesions heal, they leave thickened, scarred areas of skin. When the scalp is severely affected, there may be associated hair loss (alopecia).

People with DLE tend to be quite sensitive to the sun. They are more likely to get a sunburn, and the sun is likely to worsen their discoid lesions.

Diagnosis

Diagnosis of DLE usually requires a skin biopsy. A small sample of a discoid lesion is removed, specially prepared, and examined under a microscope. Usually, the lesion has certain microscopic characteristics that allow it to be identified as a DLE lesion. Blood tests will not reveal the type of antibodies present in SLE, and physical examination usually does not reveal anything other than the skin lesions. If antibodies exist in the blood, or if other symptoms or physical signs are found, it is possible that the discoid lesions are a sign of SLE rather than DLE.

Treatment

Treatment of DLE primarily involves the use of a variety of skin creams. Sunscreens are used for protection. Steroid creams can be applied to decrease inflammation. Occasionally, small amounts of a steroid preparation will be injected with a needle into a specific lesion. Because of their long list of side effects, steroid preparations taken by mouth are avoided. Sometimes, short-term treatment with oral steroids will be used for particularly severe DLE outbreaks. Medications used to treat the infectious disease malaria are often used to treat DLE.

Key terms

Antibody — Specialized cells of the immune system that can recognize organisms invading the body (like bacteria, viruses, and fungi). The antibodies are then able to start a complex chain of events designed to kill these foreign invaders.

Autoimmune disorder — A disorder in which the body's antibodies mistake the body's own tissues for foreign invaders. The immune system then attacks and causes damage to these tissues.

Immune system — The system of specialized organs, lymph nodes, and blood cells throughout the body that work together to defend the body against foreign invaders (bacteria, viruses, fungi, etc.).

Alternative treatment

Alternative treatments for DLE include eating a healthy diet, low in red meat and dairy products and high in fish containing omega-3 fatty acids. These types of fish include mackerel, sardines, and salmon. Following a healthy diet is thought to decrease inflammation. Dietary supplements believed to be helpful include vitamins B, C, E, and selenium. Vitamin A is also recommended to improve DLE lesions. Constitutional homeopathic treatment can help heal DLE as well as help prevent it developing into SLE.

Prognosis

For the most part, the prognosis for people with DLE is excellent. While the lesions may be cosmetically unsightly, they are not life threatening and usually do not cause a patient to change his or her lifestyle. Only about 10% of patients with DLE will go on to develop SLE.

Prevention

DLE cannot be prevented. Recommendations to prevent flares of DLE in patients with the disease include avoiding exposure to sun and consistently using sunscreen.

Resources

Organizations

The American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345. (404) 633-3777. http://www.rheumatology.org.

lupus

[loo´pus]

a name originally given to a destructive type of skin lesion, implying a local degeneration rather than systemic involvement. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. The Latin word lupus means wolf; erythematosus refers to redness. The name lupus erythematosus has been used since the 13th century because physicians thought the shape and color of the skin lesions resembled the bite of a wolf. Currently, there are at least two recognized manifestations of the disease: discoid lupus erythematosus and systemic lupus erythematosus.

chilblain lupus erythematosus a form of discoid lupus erythematosus aggravated by cold, initially resembling chilblains, in which the lesions consist of reddened infiltrated patches on the exposed areas of the body, especially the finger knuckles.

discoid lupus erythematosus (DLE) a superficial inflammation of the skin, marked by red macules up to 3 to 4 cm in width, and covered with scanty adherent scales, which extend into spreading follicles that fall off and leave scars. The lesions typically form a butterfly pattern over the bridge of the nose and cheeks, but other areas may be involved, notably the scalp and other areas that are exposed to light.

lupus erythemato´sus (LE) a group of connective tissue disorders primarily affecting women aged 20 to 40, comprising a spectrum of clinical forms in which cutaneous disease may occur with or without systemic involvement.

lupus per´nio

1. soft, purplish skin lesions on the cheeks, forehead, nose, ears, and digits, frequently associated with bone cysts, which may be the first manifestation of sarcoidosis or occur in the chronic stage of the disease.

systemic lupus erythematosus (SLE) a chronic inflammatory disease, usually febrile and characterized by injury to the skin, joints, kidneys, nervous system, and mucous membranes. It can, however, affect any organ of the body and usually has periods of remissions and exacerbations. (See plate in Dermatology Atlas.)

It was once thought that this was a fairly rare disease, but improved immunologic testing procedures have shown that it is not. It is primarily a disease of women, occurring five to ten times more often in females than in males. Although the peak incidence is between 30 and 40 years of age, the condition has also been diagnosed in the very young and the very old.

SLE is the classic prototype of autoimmune disease of connective tissue. Its etiology is unknown, but the high level of autoantibodies in persons with the condition indicates a defect in the regulatory mechanisms that sustain self-tolerance and prevent the body from attacking its own cells, cell constituents, and proteins. Patients with SLE can have a wide variety of autoantibodies against nuclear and cytoplasmic cellular components. The presence of high levels of antinuclear antibody (ANA) in SLE patients with glomerulonephritis indicates a pathogenic role for that antibody. The antibodies are directed against deoxyribonucleoprotein, DNA, histone, and a soluble non–nucleic acid molecule called Sm antigen.

Factors that appear to contribute to the development of SLE include exposure to sunlight or ultraviolet radiation from sunlamps, a genetic predisposition to the disease, certain drugs, viral infections, and hormonal influences.

Clinical manifestations of SLE are confusingly diverse owing to the involvement of connective tissue throughout the body. Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis. The detection of ANA by microscopic immunofluorescence is supportive evidence for the presence of SLE.

Either glomerulonephritis, which is usually mild, or cardiovascular manifestations such as myocarditis, endocarditis, or pericarditis, are found in about half the patients with SLE. Pulmonary disease, especially pleurisy, is also relatively common, as are gastrointestinal disturbances and lymph node involvement. Organic neurologic disturbances produce behavioral aberrations and frank psychosis in some patients; in a few others, there are peripheral neuropathies, motor weakness, and diplopia.

Supportive measures are used to prevent or minimize acute relapses and exacerbations of symptoms. The patient is instructed to avoid exposure to sunlight and ultraviolet radiation from other sources, blood transfusions, penicillin, and the sulfonamides. Active disease is treated with topical steroids, nonsteroidal antiinflammatory drugs for fever and joint pain, corticosteroids, and immunosuppressants. The goal of drug therapy is suppression of the immune system. Treatment of specific manifestations of SLE is aimed at prevention of complications. Physical therapy may be required to alleviate muscle weakness and prevent orthopedic deformities.

lupus vulga´ris the most common and severe form of tuberculosis of the skin, most often affecting the face, with formation of reddish brown patches of nodules in the corium, which progressively spread peripherally with central atrophy, causing ulceration and scarring and destruction of cartilage in involved sites.

dis·coid lu·pus er·y·the·ma·to·sus

a form of lupus erythematosus in which cutaneous lesions are present; these commonly appear on the face and are atrophic plaques with erythema, hyperkeratosis, follicular plugging, and telangiectasia; in some instances systemic lupus erythematosis may develop.

discoid lupus erythematosus (DLE)

a chronic, recurrent disease, primarily of the skin, characterized by lesions that are covered with scales and extend into follicles. The lesions are typically distributed on the face but may also be present on other parts of the body. On healing the lesions often leave atrophic, hyperpigmented, or hypopigmented scars. If hairy areas are involved, alopecia may result. The cause of the disease is not established, but there is evidence that it may be an autoimmune disorder, and some cases seem to be induced by certain drugs. It is at least five times more common in women than in men and occurs most frequently in the third and fourth decades of life. Treatment includes use of a sunblock when exposure to sunlight cannot be avoided, application of steroids to the lesions, and use of systemic antimalarial drugs such as hydroxychloroquine; systemic corticosteroid agents may be used in severe cases. See also systemic lupus erythematosus.

Discoid lupus erythematosus

dis·coid lu·pus er·y·the·ma·to·sus

(dis'koyd lū'pŭs er'ă-thē-mă-tō'sŭs)

A form of lupus erythematosus in which cutaneous lesions appear on the face and elsewhere; these are atrophic plaques with erythema, hyperkeratosis, follicular plugging, and telangiectasia.

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